Vasculitis Patient-Powered Research NetworkA partnership of the Vasculitis Clinical Research Consortium and the Vasculitis Foundation

V-PPRN Glossary of Terms

Behçet's commonly involves small to medium sized blood vessels. Males and females are affected equally but males tend to have more severe disease. Behçet's is rare in the United States. In the US, it is estimated that around 3-5/100,000 people have Behçet's. In Turkey, where it is very common, as many as 1 in 250 people have it. It is a leading cause of blindness in Israel and Japan.

Clinical Data Research Network
(CDRN)

Clinical Data Research Networks (CDRNs) are part of PCORnet. CDRNs will develop the capacity to conduct randomized comparative effectiveness studies using data from clinical practice in a large, defined population (more than one million people). These established or newly-developed networks involve two or more healthcare systems, with plans to function as integrated research network. There are currently 11 CDRNs in PCORnet.

Central Nervous System Vasculitis
(CNS)

Central nervous system (CNS) vasculitis. Central nervous system (CNS) vasculitis is a type of vasculitis that only affects the brain.

Cryogloblinemia

Cryogloblinemia is a vasculitis of small blood vessels that is caused by deposition of immune complexes: large aggregates of antibodies and the other proteins they are bound to. Some other types of vasculitis are also caused by immune complexes, but cryoglobulinemia is defined by a laboratory test that identifies immune complexes that fall out of solution in the cold. The severity of the disease is highly variable. Skin, joints, and nerves are commonly affected. Kidney disease is somewhat less common and with a wide range of severity. The heart, brain, or gastrointestinal tract each are affected in fewer than 10% of cases.

Comparative Effectiveness Research

Comparative Effectiveness Research (CER) is designed to inform health-care decisions by providing evidence on the effectiveness, benefits, and harms of different treatment options. The evidence is generated from research studies that compare drugs, medical devices, tests, surgeries, or ways to deliver health care.

Data Management and Coordinating Center

The Data Management and Coordinating Center (DMCC) houses all data for VCRC and V-PPRN.www.rdcrn.org/dmcc

De-identified Information

Information that does not identify the individual and for which there is no reasonable basis to believe the individual can be identified from it.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Eosinophilic granulomatosis with polyangiitis, also known as Churg-Strauss syndrome is a rare form of vasculitis that affects small and medium-sized vessels usually includes asthma and high levels of eosinophils in patients' blood. EGPA is one of the forms of "ANCA-associated vasculitis".

Electronic Health Records

EHR is digital documentation of an individual's medical history that is maintained by health professionals and official agencies.

Flare

A disease flare represents recurrence of inflammatory activity of the underlying vasculitis after a state of remission has been achieved with treatment. Usually disease flares are milder than the original disease presentation because the patient may still be on some immunosuppressive therapy that muffles the intensity of the inflammation or because symptoms get recognized faster by the patients and their physicians, and a renewed course of remission induction therapy is implemented earlier.

Giant cell arteritis

Giant cell arteritis (GCA) also known as "temporal arteritis" or "cranial arteritis", is a form of vasculitis affecting medium and large sized blood vessels, especially those of the aorta and arteries going from the aorta to the arms, legs and the head. GCA only affects people over the age of 50.

Granulomatosis with polyangiitis

Granulomatosis with polyangiitis (formerly known as Wegener's) (GPA) is a rare form of vasculitis mainly involving small and medium-sized blood vessels. GPA is one of the forms of "ANCA-associated vasculitis".

Health Insurance Portability and Accountability Act

HIPAA (Health Insurance Portability and Accountability Act) is a government act with the goals of reducing fraud and abuse and protect patient privacy in health care. See HIPAA Summary for more information.www.hhs.gov/ocr/privacy/hipaa/understanding/summary

Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP), also known as IgA Vasculitis, is a form of vasculitis (vessel inflammation) which affects small blood vessels. HSP is the most common form of vasculitis in children.

Microscopic polyangiitis

Microscopic polyangiitis (MPA) is a rare form of vasculitis which mainly affects small and medium-sized blood vessels. MPA is one of the forms of "ANCA-associated vasculitis".

National Patient-Centered Outcomes Research Institute (PCORnet)

Patient-Centered Outcomes Research Institute (PCORI) is supporting the development of PCORnet, the National Patient-Centered Clinical Research Network, to create a large, highly representative, national network for conducting clinical outcomes research.

PCORnet will transform clinical research by engaging patients, care providers, and health systems in collaborative partnerships to improve healthcare and advance medical knowledge. By bringing research and patient care together, this innovative health data network will be able to explore the questions that matter most to patients and their families.pcornet.org

Patient Centered Outcomes Research Institute (PCORI)

The Patient-Centered Outcomes Research Institute (PCORI) is authorized by Congress to fund and disseminate research that will provide information about the best available evidence to help patients and their healthcare providers make more informed decisions. PCORI's research is intended to give patients a better understanding of the prevention, treatment, and care options available, and the science that supports those options.www.pcori.org

The portion of an electronic health record system that is accessible to patients through a web-based application. While patient portals vary in their capabilities and functions, the V-PPRN patient portal provides ways for participants to fill out their health information, submit patient-reported data, and upload electronic health records.

Patient-powered

Patients are involved in design, development and dissemination of research.

Personal Health Information or Protected Health Information

PHI (Personal Health Information or Protected Health Information) is information about patients that is protected from inappropriate disclosure under the privacy and security mandates of the Health Insurance Portability and Accountability Act (HIPPA) of 1996 and subsequent related legislation.www.cdc.gov/mmwr/preview/mmwrhtml/m2e411a1.htm#box2

Patient-reported outcomes

PROs (patient-reported outcomes), also sometimes known as ePROs (Electronic Patient-Reported Outcomes) or PROMs (Patient-Reported Outcome Measures), capture patients' perspectives on their disease burden, their feelings, or what they are able to do as they are dealing with health conditions. PROs are often derived from a questionnaire used in a clinical trial or a clinical setting, but data are reported directly by the patient without interpretation by a clinician. Examples of instruments which measure patient-reported outcomes include PROMIS and SF-36 questionnaires.

Randomized clinical trials

A study that compares different treatments by assigning study participants by chance to separate groups; neither the researchers nor the participants can choose which group. Using chance to assign people to groups means that the groups will be similar and that the treatments they receive can be compared objectively.

Rare Diseases Clinical Research Network (RDCRN)

The Rare Diseases Clinical Research Network (RDCRN) is made up of 22 distinctive consortia and a Data Management and Coordinating Center that work together to improve treatment and outcomes for patients with rare diseases through the conduct of clinical studies, engagement of patients, and increasing the availability of information about rare diseases. The RDCRN is supported by the National Institutes of Health.www.rdcrn.org

Remission

"Remission" is a term used to indicate that there is no longer any detectable inflammatory disease activity. The use of the term "remission" implies that the disease may not be cured, and that relapses are possible in the future. Once a state of remission has been achieved the intensity of immunosuppressive therapy is usually reduced. This means that the dose of prednisone is reduced and that the first-line remission-induction agent (such as cyclophosphamide or rituximab) is replaced by better-tolerated, milder forms of immunosuppression that are used more long-term to keep the patient in remission (such as methotrexate, azathioprine, mycophenolate mofetil, or rituximab).

Steering Committee

The V-PPRN Steering Committee is responsible for oversight and strategic planning for the Network. It reviews all major activities of the Network and the Network staff. The Steering Committee is comprised of investigators, patient-partners, and staff members of the Network.

Takayasu's arteritis

Takayasu's arteritis (TAK) is a rare form of vasculitis affecting medium and large-sized arteries, primarily of the aorta (the main blood vessel that leaves the heart) and its large branches going to the arms, abdominal organs, legs and the head. Most patients with TAK are women and the disease usually starts when people are young.

Vasculitis

Vasculitis means inflammation of the blood vessels, arteries, veins or capillaries. When such inflammation occurs, it causes changes in the walls of blood vessels, such as weakening and narrowing that can progress to the point of blood vessel blockage.

A result of vasculitis is that the tissues and organs supplied by affected blood vessels do not get enough blood. This can cause organ and tissue damage that can even lead to death.

Vasculitis is a family of rare diseases that can affect people of all ages. Though some forms of vasculitis improve on their own, most require treatment. The length of treatment varies, with some people using medications for extended periods of time.

The different types of vasculitis are classified according to the size and location of the blood vessels that are affected.

Vasculitis Clinical Research Consortium (VCRC), a member of the Rare Diseases Clinical Research Network (RDCRN), is an integrated international group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis. The goal of the VCRC is to improve the care of patients with vasculitis. The VCRC is funded by the National Institutes of Health and other research partners.www.rdcrn.org/vcrc

Vasculitis Foundation (VF)

The Vasculitis Foundation is the international organization for patients with vasculitis, their families, friends and the health care professionals who care for them. The Vasculitis Foundation advocates for early diagnosis and leading edge treatment for all patients, no matter where they live. In addition, the Vasculitis Foundation partners with researchers around the world to determine the cause and discover the cure for vasculitis.www.vasculitisfoundation.org/

Vasculitis Patient-Powered Research Network

The Vasculitis Patient-Powered Research Network (V-PPRN) seeks to transform how clinical research in vasculitis is conducted by directly engaging patients, investigators, care providers, and health systems to develop research methods to electronically collect health records and patient-reported data on a large number of patients with various forms of vasculitis. The goal of the V-PPRN research program is to conduct high-quality studies that will improve the care and the health of patients with vasculitis by exploring research questions that matter most to patients and advance medical knowledge about vasculitis. The V-PPRN is funded by the Patient-Centered Outcomes Research Institute (PCORI). The V-PPRN is the only PPRN studying vasculitis.

VCRC Patient Contact Registry

The Vasculitis Clinical Research Consortium Patient Contact Registry allows individuals with vasculitis to register themselves to receive information about studies conducted by VCRC.www.rdcrn.org/vcrc/registry

V-PPRN Patient Advisory Council

The V-PPRN Patient Advisory Council (V-PAC) is a group of patient-partners who will work to develop, implement, and sustain a culture of true collaboration and partnership among patients, researchers, and other stakeholders.

V-PPRN Working Groups

The purpose of the Working Groups is to achieve a set of milestones and deliverables that address key aspects of the formation of the V-PPRN. The cross-functional Working Groups design and direct different aspects of the Network and research initiatives.